Jesse Pratt

Improved Outcomes in a Quality Improvement Collaborative for Pediatric Inflammatory Bowel Disease

OBJECTIVES: Unintended variation in the care of patients with Crohn disease (CD) and ulcerative colitis (UC) may prevent achievement of optimal outcomes. We sought to improve chronic care delivery and outcomes for children with inflammatory bowel disease by using network-based quality improvement methods. METHODS: By using a modified Breakthrough Series collaborative structure, 6 ImproveCareNow Network care centers tested changes in chronic illness care and collected data monthly. We used an interrupted time series design to evaluate the impact of these changes. RESULTS: Data were available for 843 children with CD and 345 with UC. Changes in care delivery were associated with an increase in the proportion of visits with complete disease classification, measurement of thiopurine methyltransferase (TPMT) before initiation of thiopurines, and patients receiving an initial thiopurine dose appropriate to their TPMT status. These were significant in both populations for all process variables (P < .01) except for measurement of TPMT in CD patients (P = .12). There were significant increases in the proportion of CD (55%–68%) and UC (61%–72%) patients with inactive disease. There was also a significant increase in the proportion of CD patients not taking prednisone (86%–90%). Participating centers varied in the success of achieving these changes. CONCLUSIONS: Improvements in the outcomes of patients with CD and UC were associated with improvements in the process of chronic illness care. Variation in the success of implementing changes suggests the importance of overcoming organizational factors related to quality improvement success.

Health-Related Quality of Life in Children and Adolescents With Duchenne Muscular Dystrophy

OBJECTIVES: The purpose of this study was to assess health-related quality of life (QoL) in children with Duchenne muscular dystrophy (DMD), including development and field-testing of a DMD-specific module integrated with the core Pediatric Quality of Life Inventory (PedsQL). METHODS: The PedsQL 4.0 Generic Core and DMD Module Scales were completed by 203 families, including 200 parents and 117 boys with DMD. Scores on the PedsQL Core Scales were compared with those of matched healthy children. Relationships between PedsQL scores and patient characteristics were examined. RESULTS: By both parent report and child self-report, mean PedsQL scores for boys with DMD were significantly lower than those for healthy children for physical and psychosocial QoL (P < .0001), with significantly impaired psychosocial QoL scores self-reported by 57%. Psychosocial QoL, by self-report only, tended to be higher in the older boys (13–18 years) than in younger boys (8–12 years; P = .05) and was not significantly associated with use of mobility aids. Although parents reported higher Daily Activities scores in boys receiving steroids (P = .01), boys receiving steroids reported no difference in Daily Activities but significantly less worry (P = .004). Parent–child concordance was generally in the fair to poor range. Internal consistency reliability coefficients for PedsQL DMD module scales ranged from 0.66 to 0.86. CONCLUSIONS: Overall, boys with DMD reported significantly lower QoL than their healthy peers. Despite decreased physical functioning, older boys seem to perceive better psychosocial QoL than perceived by their parents and by younger boys, unrelated to their need for mobility aids.

Variation in Growth of Infants with a Single Ventricle

OBJECTIVE The study goal was to evaluate interstage growth variation among sites participating in the National Pediatric Cardiology Quality Improvement Collaborative registry caring for infants with hypoplastic left heart syndrome and to identify nutritional practices common among sites achieving best growth outcomes. STUDY DESIGN This was a retrospective analysis of infants in the registry who had presented due to their superior cavopulmonary connection (SCPC) and whose surgical site had enrolled ≥ 4 eligible patients in the registry. The primary outcome variable was weight-for-age z-score (WAZ) change between Norwood discharge and presentation for SCPC (interstage period). Blinded, structured interviews were performed with each site regarding site-specific nutritional practices. Practices common among sites with positive interstage WAZ changes were identified. RESULTS Sixteen centers enrolled 132 infants from December 2008 through December 2010. Median age at SCPC was 5 months (2.6-12.6), and median interstage WAZ change was -0.29 (-3.2 to 2.3). Significant variation in WAZ changes among sites was demonstrated (P < .001). Sites that used standard feeding evaluation prior to Norwood discharge and that closely monitored for specific weight gain/loss red flags in the interstage period demonstrated significantly better patient growth than those that did not use these practices (P = .002). CONCLUSIONS Considerable variation exists in interstage growth among patients receiving care at these 16 surgical sites. Standardization of interstage nutritional management with focus on best nutritional practices may lead to improved growth in this high-risk population of infants.

Usefulness of combined history, physical examination, electrocardiogram, and limited echocardiogram in screening adolescent athletes for risk for sudden cardiac death.

Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for high-risk cardiac defects is controversial. The purpose of this study was to assess the utility and feasibility of a comprehensive cardiac screening protocol in an adolescent population. Adolescent athletes were recruited from local schools and/or sports teams. Each subject underwent a history and/or physical examination, an electrocardiography (ECG), and a limited echocardiography (ECHO). The primary outcome measure was identification of cardiac abnormalities associated with an elevated risk for sudden death. We secondarily identified cardiac abnormalities not typically associated with a short-term risk of sudden death. A total of 659 adolescent athletes were evaluated; 64% men. Five subjects had cardiac findings associated with an elevated risk for sudden death: prolonged QTc >500 ms (n = 2) and type I Brugada pattern (n = 1), identified with ECG; dilated cardiomyopathy (n = 1) and significant aortic root dilation; and z-score = +5.5 (n = 1). History and physical examination alone identified 76 (11.5%) subjects with any cardiac findings. ECG identified 76 (11.5%) subjects in which a follow-up ECHO or cardiology visit was recommended. Left ventricular mass was normal by ECHO in all but 1 patient with LVH on ECG. ECHO identified 34 (5.1%) subjects in whom a follow-up ECHO or cardiology visit was recommended. In conclusion, physical examination alone was ineffective in identification of subjects at elevated risk for SCDY. Screening ECHO identified patients with underlying cardiac disease not associated with immediate risk for SCDY. Cost of comprehensive cardiac screening is high.

Left ventricular noncompaction in Duchenne muscular dystrophy

BackgroundLeft ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.MethodsCardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.ResultsLVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.ConclusionThe high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

Prevalence and patterns of retention in cardiac care in young adults with congenital heart disease

The population with adult congenital heart disease is expanding. Cardiac care retention and follow-up patterns were assessed in 153 adults with congenital heart disease (median age, 24.5 years), previously compliant as teenagers. The majority (125; 81.7%) were retained in care, most often by a pediatric cardiologist (69%). The rate of retention was surprisingly high.

Postoperative Assessment of Laryngopharyngeal Dysfunction in Neonates After Norwood Operation

BACKGROUND The purpose of this study was to evaluate the incidence of vocal cord (VC) and swallowing dysfunction in infants after the Norwood operation and to examine the relationship between laryngopharyngeal dysfunction and postoperative outcomes. METHODS We conducted a retrospective review of 63 infants who underwent routine postoperative fiberoptic endoscopic evaluation of swallowing function and vocal cords after a Norwood operation at our institution during a recent 6-year period (2003-2009). RESULTS The overall incidence of VC dysfunction after the Norwood operation was 58.7%. After a modification of the aortic arch dissection technique in 2007, the incidence of VC dysfunction decreased significantly from 79.5% in 2003 through 2006 to 25% in 2007 through 2009 (p<0.001). The incidence of swallowing dysfunction also decreased from 23.1% in 2003 through 2006 to 4.2% in 2007 through 2009 (p=0.07). Swallowing dysfunction was more common in patients with VC dysfunction (21.6%) as compared with patients without VC dysfunction (7.7%; p=0.18). Patients with VC dysfunction were more often discharged home on tube-only feeding regimens compared with infants without VC dysfunction (46% versus 26.9%). In infants with both VC and swallowing dysfunction, 75% were discharged exclusively to have tube feeding. Median hospital length of stay tended to be longer in infants with swallowing dysfunction (31 days) than in infants without swallowing dysfunction (23 days; p=0.16). CONCLUSIONS Vocal cord and swallowing dysfunction are common in infants after the Norwood operation and may increase the need for tube feeding regimens. Modification of surgical techniques for dissection and mobilization of the aorta can significantly reduce the incidence of these adverse outcomes.

Role of sex in the treatment and clinical outcomes of pediatric patients with inflammatory bowel disease.

Objective: To examine sex differences in medical therapy and clinical outcomes in pediatric patients with inflammatory bowel disease (IBD). Methods: We performed a cross-sectional analysis of children with Crohn disease (CD) and ulcerative colitis (UC) using data from the ImproveCareNow Network collected between May 2007 and May 2010. Clinical remission, disease severity, body mass index (BMI) z scores, normal height velocity, and medication use were analyzed by sex and age. Results: One thousand four hundred nine patients were included (993 had CD and 416 had UC). No significant sex differences were found in disease severity, BMI, height velocity, or use of medications. Further analysis of combination therapy with infliximab + 6-mercaptopurine/azathioprine and infliximab + methotrexate also did not reveal any differences. No sex differences were found after mediation use was stratified by age (those younger than 13 years and those 13 years old or older). Conclusions: In this sample of CD and UC pediatric patients, no significant sex differences were found in disease severity, BMI, height velocity, or medication use. Our data do not support the use of sex as a major factor in patient risk stratification for children with IBD. In addition, despite concerns for sex-specific complications of some medications, our analysis did not suggest any sex differences in medication use.

Relationship between echocardiographic LV mass and ECG based left ventricular voltages in an adolescent population: related or random?

In attempts to detect diseases that may place adolescents at risk for sudden death, some have advocated for population‐based screening. Controversy exists over electrocardiography (ECG) screening due to the lack of specificity, cost, and detrimental effects of false positive or extraneous outcomes.

The impact of the 2009 H1N1 influenza pandemic on pediatric patients with sickle cell disease.

Respiratory infections are associated with clinically significant illness in patients with sickle cell disease (SCD). The 2009 H1N1 pandemic was perceived as a significant threat to this population.